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PubMedDate not supplied

[Constipation in children].

Constipation is a common disease in paediatric age, with an incidence ranging from 0.3 to 8% in paediatric patients, and from 10 to 25% among paediatric gastroenterological patients. In 90-95% of cases constipation is a functional, and often due to an exclusively milky diet or, in advanced age, to an inadequate fibres intake. Among the organic forms causing constipation, especially in new-born age, Hirshsprung disease, anorectal malformations, intestinal atresiae and stenosis are frequent. Moreover, recent studies have shown that constipation is often the symptom of a cow's milk proteins intolerance, that leadis to colorectal mucosa inflammation, with peristalsis decrease and fecal slackness. In these patients a milk's proteins free diet recovers constipation. In most persistent forms, total intestinal transit time (TITT), anorectal manometry, sphynteric muscles electromyografy and defecofraphy are useful to the diagnosis. In more than 90% of cases simple diet revisions, fecal softening, evacuative suppositories and enemas recovers constipation, some times a psychological approach is useful. Furthermore, excellent results can be obtained by giving low doses of polietiltnglycol (PEG), which has been recently introduced for the treatment of functional chronic constipation.

Child↗
PubMed1987

Isolation of the right subclavian artery with subclavian steal in a child with Klippel-Feil anomaly: an example of the subclavian artery supply disruption sequence.

We studied a patient with Klippel-Feil anomaly and subclavian steal due to isolation of the right subclavian artery. Other anomalies included hypoplasia of the right clavicle and right vertebral artery, low bifurcation of the left common carotid artery and left choanal atresia. The patient was mentally retarded with corticospinal, cerebellar, and brain stem signs. The entire brain and cervical spinal cord were small; there was no central nervous system malformation, hydrocephalus, or bony impingement on neural structures. Embryologic vascular disorders have been found to be responsible for various congenital systemic and neurologic anomalies such as intestinal atresia and hydranencephaly. Subclavian artery supply disruption sequence has been hypothesized to result in Klippel-Feil, Poland, and Möbius anomalies. In this case the vertebral and facial abnormalities may well be related to anomalous subclavian and carotid supply. An embryonic vascular "steal" has been thought to cause the sirenomelia anomaly. We postulate that the subclavian steal, during the embryonic period, may have been responsible for the small size of this patient's cerebral hemispheres, brain stem, cerebellum, and cervical spinal cord.

Adolescent↗
PubMedDate not supplied

[Nutritional support in short bowel syndrome].

Short bowel syndrome most commonly result after bowel resection for necrosis of the bowel. It may be caused by arterial or venous thrombosis, volvolus and in children, necrotizing enterocolitis. The other causes are Crohn's disease, intestinal atresia. The factors influencing the risk on short bowel syndrome are the remaining length of the small bowel, the age of onset, the length of the colon, the presence or absence of the ileo-coecal valve and the time after resection. Besides nutritional deficiencies there some other consequences of extensive resections of the small intestine (gastric acid hypersecretion, d-lactic acidosis, nephrolithiasis, cholelithiasis), which must be diagnosed, treated, and if possible, prevented. With current therapy, most patients with short bowel have normal body mass index and good quality of life.

Humans↗
PubMed1991

Short-bowel syndrome in children. Quality of life in an era of improved survival.

A number of disorders in childhood can result in short-bowel syndrome (small bowel length, less than 100 cm). Improved care has increased survival in patients with short-bowel syndrome, but the quality-of-life factors associated with such improved survival have not been examined, to our knowledge. Sixteen consecutive pediatric patients with short-bowel syndrome (bowel length range, 22 to 98 cm) were followed up for 2 to 10 years. The original diagnoses were as follows: necrotizing enterocolitis (n = 6), multiple intestinal atresias (n = 4), extensive aganglionosis (n = 2), meconium peritonitis (n = 2), and midgut volvulus (n = 2). The range of initial hospitalization was from 62 to 395 days, and 13 of 16 patients have required readmission (two to 14 times). All patients required multiple operations (range, two to 14 operations), including combinations of venous access, adhesiolysis, tapering enteroplasty, reversed intestinal segments, and pull-through procedure. Nine of 16 patients received home total parenteral nutrition, and 12 of 16 patients required home elemental diets, usually via pump feedings. Fifteen patients (94%) survived. Two survivors are deaf, and one of these has mild developmental delay. Seven survivors (age range, 6 to 10 years) attend a regular school, four while receiving total parenteral nutrition or an elemental diet. Ten of 15 survivors are off all nutritional support (including the child with a 22-cm small bowel), with four others weaning. The presence or absence of an ileocecal valve did not affect outcome. Modern nutritional support methods provide excellent survival and quality of life for children with short-bowel syndrome.

Child↗
PubMed1985

Septa in the appendix: a previously undescribed condition.

A previously undescribed condition of the appendix, consisting of complete and incomplete septa is reported in 25 cases. The abnormality occurred in persons younger than 30 years. In all cases the lesion was found to be associated with acute appendicitis. Possible factors discussed with respect to aetiology and pathogenesis are: a congenital abnormality similar to intestinal atresia; post-inflammatory fusion of ulcerated, swollen mucosal folds; ischaemia caused by thrombosed vessels; mucosal folding in the process of expulsion of appendiceal contents.

Adolescent↗
PubMed1995

Stapled intestinal anastomosis in neonates and infants: use of the endoscopic intestinal stapler.

Stapled anastomoses of the gastrointestinal tract, commonplace in adults and older children, are rarely performed in newborns because of the size of the instruments. The development of smaller stapling devices for laparoscopic and thoracoscopic procedures presented an opportunity to evaluate the efficacy of such anastomoses in neonates and infants. Over an 11-month period, seven newborn or young infants underwent stapled functional end-to-end small or large intestinal anastomoses using the Endo-GIA 30. The male:female ratio was 3:4, and the mean age was 72d (range, 1 to 134 days). The mean weight at the time of the stapled anastomosis was 3.7 kg (range, 1.88 to 6.6 kg). The clinical setting requiring the intestinal anastomoses included ileostomy closure after perforated necrotizing enterocolitis (3), intestinal atresia (1), distal ileal volvulus (1), left colon mesenteric cyst (1), and biliary atresia (1). There were no deaths or anastomotic leaks. Nasogastric decompression was discontinued at a mean of 6 days (range, 3 to 11 days), and oral feedings were begun at a mean of 7.0 days (range, 4 to 12 days). The patients were discharged from the hospital 7 to 117 days after the operation (mean, 33 days). The endoscopic stapling instrument is safe and effective in performing small and large intestinal anastomoses in newborns and young infants.

Age Factors↗
PubMed1996

Intestinal obstruction caused by duplication of the caecum.

Two cases of caecal duplication are presented, one in a neonate and one in an infant. The diagnosis was made at laparotomy, which had been undertaken for the presumptive diagnosis of intestinal atresia and torsion of an ovarian cyst respectively. Also the literature on alimentary tract duplications is reviewed, referring to the incidence, presenting symptoms, and location of the duplication, in particular that of the caecum.

Cecum↗
PubMed1984

Functional capability of blind small bowel loops after intestinal remodelling techniques.

An experimental model was developed in mongrel puppies to simulate the dilation and hypertrophy found in intestinal atresia. The diameter of the dilated segment was reduced either by antimesenteric resection or by antimesenteric infolding techniques. This study indicates that the infolding technique is better, both structurally and functionally, following artifically induced dilation.

Animals↗
PubMedDate not supplied

[Surgical malabsortion (author's transl)].

Two interesting aspects should be considered in intestinal malabsortion from the Surgical point of view. First, this sindrome may be caused by subobstructive lesions of the gut, which may be able of surgical correction. Second, malabsortion may follow certain operations performed in the gastrointestinal tract. Many of this problems are connected with alterations of the intestinal flora, and or a diminution in the obstructive intestinal surface. The clinical picture is not clear due to the pathological and surgical implications. The experience of the last 10 years is reviewed and conected with the following items, whem accompanied by not infectious diarrhea: a) Hiatus hernia and vaguectomy; b) Gastrointestinal by-pass; c) Intestinal atresia and stenosis; d) Small gut resections-Short bowel sindrome; e) Incompetence of the ileocecal valve.

Child, Preschool↗
PubMed1997

The spectrum of congenital anomalies of the VATER association: an international study.

The spectrum of the VATER association has been debated ever since its description more than two decades ago. To assess the spectrum of congenital anomalies associated with VATER while minimizing the distortions due to small samples and referral patterns typical of clinical series, we studied infants with VATER association reported to the combined registry of infants with multiple congenital anomalies from 17 birth defects registries worldwide that are part of the International Clearinghouse for Birth Defects Monitoring Systems (ICB-DMS). Among approximately 10 million infants born from 1983 through 1991, the ICB-DMS registered 2,295 infants with 3 or more of 25 unrelated major congenital anomalies of unknown cause. Of these infants, 286 had the VATER association, defined as at least three of the five VATER anomalies (vertebral defects, anal atresia, esophageal atresia, renal defects, and radial-ray limb deficiency), when we expected 219 (P<0.001). Of these 286 infants, 51 had at least four VATER anomalies, and 8 had all five anomalies. We found that preaxial but not other limb anomalies were significantly associated with any combination of the four nonlimb VATER anomalies (P<0.001). Of the 286 infants with VATER association, 214 (74.8%) had additional defects. Genital defects, cardiovascular anomalies, and small intestinal atresias were positively associated with VATER association (P<0.001). Infants with VATER association that included both renal anomalies and anorectal atresia were significantly more likely to have genital defects. Finally, a subset of infants with VATER association also had defects described in other associations, including diaphragmatic defects, oral clefts, bladder exstrophy, omphalocele, and neural tube defects. These results offer evidence for the specificity of the VATER association, suggest the existence of distinct subsets within the association, and raise the question of a common pathway for patterns of VATER and other types of defects in at least a subset of infants with multiple congenital anomalies.

Abnormalities, Multiple↗
PubMed2026

Stapled versus hand-sewn intestinal anastomosis in pediatric surgery: A systematic review and meta-analysis.

BACKGROUND: Intestinal anastomosis is a core procedure in pediatric gastrointestinal surgery, performed for conditions such as necrotizing enterocolitis, intestinal atresia, Hirschsprung's disease, and inflammatory bowel disease. Although stapled anastomosis (SA) may improve operative efficiency, its safety and effectiveness compared with hand-sewn anastomosis (HA) in children remain uncertain. This meta-analysis compared clinical outcomes of SA and HA in pediatric patients. METHODS: The study followed PRISMA guidelines and was prospectively registered in PROSPERO (CRD420251177257). A systematic search of PubMed, Dimensions, and the Cochrane Library was conducted through June 2025. Studies including children under 7 years undergoing intestinal SA or HA were eligible. Two reviewers independently performed study selection, data extraction, and risk-of-bias assessment using ROB 2 and ROBINS-I tools. Statistical analysis was conducted using Comprehensive Meta-Analysis software (v3) with a random-effects model. RESULTS: Eleven studies involving 903 patients met inclusion criteria, including two randomized controlled trials. Of these, 333 patients underwent SA and 570 underwent HA. SA was associated with significantly shorter operative time (mean difference [MD] = -19.26 min; 95% CI: -24.24 to -14.28; p < 0.001) and earlier initiation of oral feeding (MD = -2.32 days; 95% CI: -3.78 to -0.86; p = 0.002). No significant differences were found in anastomotic leakage, stricture formation, reoperation rate, or length of hospital stay. CONCLUSIONS: Stapled anastomosis appears as safe as hand-sewn techniques in pediatric intestinal surgery while offering shorter operative duration and faster postoperative feeding recovery. Selective use of stapled techniques is supported when anatomically feasible, though further multicenter randomized trials are needed.

Humans↗
PubMed1998

Short bowel syndrome in patients studied at the National Institute of Pediatrics in Mexico. Care, cost and perspectives.

BACKGROUND: Short bowel syndrome (SBS) continues to be one of the most challenging problems in pediatric surgery. Intestinal transplantation (IT) seems to be the best form of treatment for this pathology. However, it is thought that the development of an IT program may be more expensive than the present manner of treatment. METHODS: To assess this item, and to identify potential candidates for IT, we reviewed the charts of all the patients with SBS treated at our Institute from 1989 to 1994. RESULT: Nine patients were identified as carriers of SBS; six with intestinal atresia, two with midgut volvulus and one with post-traumatic mesenteric thrombosis. The small bowel remnants varied from 1 to 80 cm, seven patients had remnants shorter than 30 cm, and the ileocecal valve was resected in three. RESULTS: The overall morbidity and mortality was extremely high; four patients died within the first 3 months postresection and those still alive have had several complications: sepsis; hydroelectrolyte imbalances secondary to loose stools; thrombosis or infection of the catheter; TPN-related cholestasis, and malabsorption syndromes, etc. No patient survived with an intestinal remnant shorter than 15 cm. Of the five survivors, four have a weight/age deficit greater than 40%, two have rickets, one still depends on TPN and all, except one, require special enteral diets. Multiple central venous accesses had to be performed in every patient (mean 4.8). They all required multiple readmissions and have spent a considerable part of their lives as inpatients. The mean of the calculated cost per patient was $50,000 USD, while the minimal wage in Mexico is $1,616 USD/year (1). CONCLUSIONS: The shorter the segment of the retained bowel and the longer the survival, the higher the cost. These results may be further improved with the development of IT and, probably, with the same economic burden.

Child↗
PubMed2006

Gastroschisis in the United States 1988-2003: analysis and risk categorization of 4344 patients.

OBJECTIVE: Gastroschisis is a rare congenital abdominal wall defect through which intraabdominal organs herniate and it requires surgical management soon after birth. The objectives of this study were to profile patient characteristics of this anomaly utilizing data from two large national databases and to validate previous risk stratification categories of infants born with this condition. METHODS: An analysis was performed using 13 years of the National Inpatient Sample database (1988-1996, 1998, 1999, 2001, 2002) and 3 years of the Kids' Inpatient Database (1997, 2000, 2003). These combined databases contain information from nearly 93 million discharges in the United States. Infants with gastroschisis were identified by International Classification of Disease-9 procedure code 54.71 (repair of gastroschisis) and an age at admission of <8 days. Variables of gender, race, geographic region, co-existing diagnoses, length of stay, hospital charges adjusted to 2005 dollars, complications and inpatient mortality were collected from the databases. Infants were divided into simple and complex categories based on the absence or presence of intestinal atresia, stenosis, perforation, necrosis or volvulus. Comparisons between groups were performed using Pearson's chi (2) for categorical outcomes and the Kruskal-Wallis test for non-normally distributed continuous variables. RESULTS: A total of 4344 infants with gastroschisis were identified. These were comprised of 44.0% female infants (n=1910), 46.4% male infants (n=2017) whereas 9.6% were not reported (n=415). Racial analysis showed the largest subset being white in 40.9% of infants (n=1775) with Hispanic infants being the next highest group reported at 17.2% (n=745). Co-existing intestinal anomalies were the most common, affecting 9.9% (n=429) infants, whereas certain cardiac (6.8%, n=294) and pulmonary (1.7%, n=72) conditions were also identified. Simple gastroschisis represented 89.1% (n=3870) of the group whereas 10.9% (n=474) were complex in nature. Simple and complex patients differed in median length of stay (28 vs 67 days, P<0.01), inpatient mortality (2.9 vs 8.7%, P<0.01) and median inflation-adjusted hospital charges (90,788 dollars vs 197,871 dollars, P<0.01). CONCLUSIONS: These data represent a national analysis of the largest group of infants with gastroschisis to date which further aids the characterization and understanding of this serious congenital condition.

Female↗
PubMed2000

Isolated liver transplant and sequential small bowel transplantation for intestinal failure and related liver disease in children.

Liver dysfunction is a well-recognized complication of intestinal failure in children. Advances in total parenteral nutrition (TPN) have allowed these children to survive while their intestinal tract gradually adapts. Unfortunately TPN may lead to cholestatic liver disease particularly in the young children. Progression of liver disease is associated with a poor prognosis and is an indication for small bowel transplantation. We report our experience of orthotopic liver transplantation in four children with short gut and sequential liver and small bowel transplantation in one child. All children had TPN-related liver failure. Causes of intestinal failure included necrotising enterocolitis (n=2), gastroschisis (n=1), intestinal atresia (n=1), and megacystic, microcolon syndrome (n=1). At the time of liver transplantation the children's mean age was 10.9 months (2.5-24) and weight 6.7 kg (4.8-10.1). The mean serum bilirubin was 522 micromol/liter (299-823), aspartate transaminase 423 IU/liter (49-1024) and international normalized ratio 2.8 (2-3.9). There were two deaths both from respiratory failure secondary to adenovirus pneumonia including the child who received a sequential small bowel transplant. Three children with isolated liver grafts are alive and off TPN at 20 months (mean) follow up (range 6-35). Isolated orthotopic liver transplantation has a role in selected children with intestinal failure, particularly those with short but normally functioning gut and progressing with satisfactory intestinal adaptation but developing liver disease. Those children with TPN-related liver disease and unadapted gut or irreversible intestinal disease need combined liver and small bowel transplantation. Sequential small bowel transplantation is feasible after orthotopic liver transplantation and may provide an option for the child with terminal liver and small bowel failure.

Adolescent↗
PubMed2004

Three-year experience in clinical intestinal transplantation.

BACKGROUND: The purpose of this study was to evaluate the outcome of 19 patients who underwent intestinal transplantation (ITx) for intestinal failure. METHODS: The 19 patients who underwent primary ITx between December 2000 and May 2003 were prescribed three different immunosuppressive protocols that included daclizumab, alemtuzumab, and antithymocyte globulin induction, respectively. A mucosal surveillance protocol for early detection of rejection consisted of zoom video endoscopy and serial biopsies associated with orthogonal polarization spectral imaging. Retrospective review of the clinical records was performed to assess the impact of new modalities of immunosuppression and intestinal mucosal monitoring on patient outcomes. RESULTS: All patients were adults (mean age 35.8 years). Etiology of intestinal failure included chronic intestinal pseudo-obstruction (n = 6), intestinal angiomatosis (n = 1), Gardner syndrome (n = 2), intestinal infarction (n = 8), radiation enteritis (n = 1), and intestinal atresia (n = 1). All patients experienced complications from total parenteral nutrition (TPN). Thirteen patients (68.4%) received isolated small bowel, whereas six (31.6%) received multivisceral grafts with or without the liver. Thirteen of 19 patients experienced at least one episode of rejection (68.4%). Most ACR episodes were treated with steroid boluses and resolved completely within 5 days. The overall 1-year patient survival was 82%. All living patients are in good health with functioning grafts having been weaned off TPN after a mean of 23.7 days post-ITx. DISCUSSION: Advances in immunosuppressive therapy with early detection and prompt treatment of rejection episodes make ITx a valuable treatment option for patients with intestinal failure and TPN-related life-threatening complications.

Adult↗
PubMed2000

Meconium peritonitis in utero.

To clarify the relationship between clinical features in utero and postnatal prognosis, 20 fetuses who underwent ultrasonic (US) evaluation for meconium peritonitis (MP) over a 17-year period were reviewed. According to final US findings in utero, patients were classified into three types. Type I (massive meconium ascites) was noted is 5 cases, type II (giant pseudocyst) in 4, and the other 11 were classified as type III (calcification and/or small pseudocyst). Abdominal calcifications were identified in only 5 cases (2 type I, 1 type II, 2 type III). Seven fetuses who had associated polyhydramnios (1 Type I, 1 Type II, 2 Type III) and fetal hydrops (3 Type II) were delivered before 36 weeks' gestation. Cardiopulmonary resuscitation at birth was required in 9 cases (5 type I, 4 type II) who underwent abdominal drainage before delivery and/or immediately after birth. Although dilatation of the intestine was identified in 10 fetuses (2 type II, 8 Type III), 18 had intestinal atresia and 2 had fecal obstruction of the distal ileum. Four infants (2 type I, 1 type II, 1 type III) died of respiratory failure and postoperative complications. These results indicated that careful fetal US may be useful for perinatal management of MP.

Ascites↗
PubMed2005

Matrilysins-1 and -2 (MMP-7 and -26) and metalloelastase (MMP-12), unlike MMP-19, are up-regulated in necrotizing enterocolitis.

OBJECTIVES: Necrotizing enterocolitis (NEC) is the most common gastrointestinal disease of premature infants characterized histologically by extensive tissue injury and inflammation. Matrix metalloproteinases (MMP) are involved in tissue remodeling and cell migration, both being important aspects of inflammatory disease. The aim of this study was to investigate whether MMPs play a role in the pathogenesis of NEC. METHODS: Expression of MMP-1, -7, -9, -10, -12, -19 and -26 was studied using in situ hybridization/immunohistochemistry in samples intestinal tissue removed from 15 patients with NEC; in 7 of them control samples were obtained at closure of stomas. Six intestinal samples from patients with intestinal atresia and four samples of necrosis were also included in the material examined. Laminin-5 was immunostained to find migrating enterocytes and cytokeratin to delineate mucosal epithelium. RESULTS: MMP-7 protein was upregulated in the epithelium of 12/18 NEC samples. MMP-26 was induced in stromal cells of 12/17 NEC specimens. Stromal expression was found for MMP-1 and -12 mRNAs in 7/18 samples. MMP-1 was also detected in the epithelium of regenerating areas. Both NEC and stoma samples expressed MMP-9 in inflammatory cells. Epithelial MMP-19 was downregulated in NEC. CONCLUSIONS: Our results suggest that several MMPs may be major factors in tissue destruction and remodeling in NEC. Targeted inhibition of matrilysins, using synthetic MMP inhibitors or blockers of their signal transduction pathways, may represent a novel therapeutic option for the treatment of intestinal inflammation associated with NEC.

Case-Control Studies↗
PubMed1985

Longitudinal division of small intestine: a surgical possibility for children with the very short bowel syndrome.

Surgical approach to short bowel syndrome has been dealing with two major problems: lack in absorptive surface and dysfunction of the peristalsis of the widely distended loop above the anastomosis. In those children having a very short intestine, one is reluctant to either resect or reduce the diameter of this loop. Bianchi, followed by Boeckman and Traylor, described a procedure of loop lengthening by dividing it longitudinally. Their procedure has the advantage of restoring normal peristalsis without losing any absorptive surface. A modification of the original procedure of Bianchi is described. We report on its application in a child born with laparoschisis and intestinal atresia; she had in fact 25 centimetres of duodenum and proximal jejunum anastomosed with left colonic angle. This child was referred to us with functional occlusion related to distension above an intact and unobstructed anastomosis. She was operated on at six weeks of age. Postoperatively oral feeding could be started after one month. Broviak's catheter for parenteral nutrition was removed at six months. In conclusion we believe that this technique offers a chance of better and faster adaptation to children born with short bowel syndrome.

Dietary Fats↗